Ultrastructure of myocardium in the Hurler syndrome. Possible relation to cardiac function.
Cardiac tissues obtained at post mortem examination of eight patients with the Hurler syndrome, who ranged in age from 5 to 23 years, were examined by histochemical methods and electron microscopy. Extensive myocardiocytic vacuolization and increased interstitial fibrous tissue were noted by light microscopy in all hearts. The cytoplasmic (perinuclear) vacuoles contained Luxol-fast-blue-positive substance. At the ultrastructural level, abnormal cytoplasmic organelles were present within the myocardiocytes in all patients. These organelles were of three types: zebra bodies (ZB), membranous cytoplasmic bodies (MCB) and granulomembranous bodies (GMB). As ZB and MCB are believed to represent the morphological counterpart of accumulated gangliosides, these substances rather than glycosaminoglycans appear to be stored within myocardiocytes of patients with the Hurler syndrome. The accumulation of gangliosides and the consequent damage to the myocardial substratum probably contributes to the clinically evident cardiac disease, so often observed in the patients with this disorder.
D000293:Adolescent; D000328:Adult; D001344:Autopsy; D002648:Child; D002675:Child, Preschool; D005260:Female; D005732:Gangliosides / Q000032:analysis; D006801:Humans; D008297:Male; D008854:Microscopy, Electron; D008059:Mucopolysaccharidosis I / Q000473:pathology; D009206:Myocardium / Q000473:pathology / Q000648:ultrastructure*; D014617:Vacuoles / Q000648:ultrastructure
D005732:Gangliosides